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Abernethy Malformation
Alexander J. Towbin, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Congenital extrahepatic portosystemic shunt
              • Definitions

                • Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
                  • Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
                    • Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
                    • Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
                  • Type 2: Intact but hypoplastic intrahepatic PV with extrahepatic portosystemic shunt

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Angiographic Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Baiges A et al: Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 71(2):658-69, 2020
                                        2. Bueno J et al: Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts. J Pediatr Surg. 55(9):1897-902, 2020
                                        3. DiPaola F et al: Congenital portosystemic shunts in children: associations, complications, and outcomes. Dig Dis Sci. 65(4):1239-51, 2020
                                        4. Rajeswaran S et al: Abernethy malformations: evaluation and management of congenital portosystemic shunts. J Vasc Interv Radiol. 31(5):788-94, 2020
                                        5. Sokollik C et al: Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 56(6):675-81, 2013
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Synonyms

                                                      • Congenital extrahepatic portosystemic shunt
                                                    • Definitions

                                                      • Congenital portosystemic shunt characterized by variable absence of intrahepatic portal vein (PV)
                                                        • Type 1: Absence of intrahepatic PV with extrahepatic portosystemic shunt
                                                          • Type 1a: Splenic vein & superior mesenteric vein drain separately into systemic vein
                                                          • Type 1b: Splenic vein & superior mesenteric vein form common trunk before draining into systemic vein
                                                        • Type 2: Intact but hypoplastic intrahepatic PV with extrahepatic portosystemic shunt

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Angiographic Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Baiges A et al: Congenital extrahepatic portosystemic shunts (Abernethy malformation): an international observational study. Hepatology. 71(2):658-69, 2020
                                                                              2. Bueno J et al: Radiological and surgical differences between congenital end-to-side (Abernethy malformation) and side-to-side portocaval shunts. J Pediatr Surg. 55(9):1897-902, 2020
                                                                              3. DiPaola F et al: Congenital portosystemic shunts in children: associations, complications, and outcomes. Dig Dis Sci. 65(4):1239-51, 2020
                                                                              4. Rajeswaran S et al: Abernethy malformations: evaluation and management of congenital portosystemic shunts. J Vasc Interv Radiol. 31(5):788-94, 2020
                                                                              5. Sokollik C et al: Congenital portosystemic shunt: characterization of a multisystem disease. J Pediatr Gastroenterol Nutr. 56(6):675-81, 2013